Abstract
Introduction. Clival chordomas represent a rare but clinically significant subset of skull base tumors, characterized by a locally aggressive nature and a location in proximity to vital neurovascular structures. Surgical resection, often combined with adjuvant therapies, remains the cornerstone of clival chordoma treatment, and various approaches and techniques have evolved to maximize tumor removal while preserving neurological function. Recent advancements in skull base surgery, imaging, and adjuvant therapies have improved outcomes by reducing morbidity and thus enhancing long-term survival. Methods and Results. We have conducted a systematic review on PubMed/Medline following PRISMA guidelines regarding indications, the extent of resection (EOR), and complication rates. Then, we present three illustrative cases from our personal experience, which started 25 years ago with CVJ instrumentation procedures and 15 years ago with anterior decompressive transmucosal procedures performed with the aid of an operative microscope, an endoscope, and neuroradiological monitoring. Conclusions. Traditionally, the transoral approach (TOA) is the most frequently used corridor for accessing the lower clivus and the anterior craniovertebral junction (CVJ), without the need to mobilize or retract neural structures; however, it is associated with a high rate of complications. The endonasal approach (EEA) provides access to the anterior CVJ as well as to the lower, middle, and superior clivus, decreasing airway and swallowing morbidity, preserving palatal function, decreasing postoperative pain, and reducing the incidence of tracheostomy. The submandibular retropharyngeal approach (SRA) allows unique access to certain cervical chordomas, which is better suited when the lesion is located below the clivus and in the midline.